Download Two Case Reports of Situs Inversus Totalis

Case Report
Copyright©2013, Iranian society of anatomical sciences. All rights reserved.
Downloaded from anatomyjournal.ir at 8:27 +0430 on Saturday May 13th 2017
Two Case Reports of Situs Inversus Totalis
Reza Ahadi, M.Sc.1*, Hadi Shamshirband, B.Sc.2
1. Shaheed Beheshti University of Medical Sciences and Medical Imaging Center, Shohadaye HafteTir Hospital, Shahid Rajaei St, Tehran, Iran
2. Department of Radiology Tehran University of Medical Sciences, Tehran, Iran
*Corresponding author, E‐mail address: [email protected] Received: January 2013
Accepted: April 2013
Reza Ahadi has a BSc in Radiology and MSc in Anatomical Sciences. Currently, he is a
PhD student at the Neuroscience Research Center of Shahid Beheshti University of
Medical Sciences. He is a staff member of the Medical Imaging Center at Shohadaye
Hafte-Tir Hospital in Tehran. Mr. Ahadi is a member of the Iranian Association of
Neurosciences and is an expert in medical imaging and computed tomography scans.
His special interest is the neuroimaging of traumatic brain and spinal cord injuries and
biomarkers in humans.
Abstract
Case A, a 27-year-old Iranian male and case B, a 22-year-old Afghani male, both presented to the Emergency
Department of Shohadaye Hafte-Tir Hospital with multiple traumas due to car accidents. Neither was aware of his
unusual anatomy. One case unaware two just right heart. Both patients were referred to the Imaging Department for
CT scans and some radiographic studies. We discovered they were a situs inversus totalis cases with right heart in
side and left in the side liver. in CT scan of the thorax and abdomen for assessment of traumatic injuries. Thoracic
and abdominal organs and the viscera were inversed complementary. It is important to inform medical personnel of
the diagnosis of situs inversus totalis in order to decrease errors and prevent complications that arise from patient
assessment and care, particularly in cases of appendicitis and abdominal organ lacerations.
Key words: Situs Inversus Totalis, Congenital Abnormalities, Computed Tomography, X-Ray
To cite this paper: Ahadi R, Shamshirband H. Two Case Reports of Situs Inversus Totalis. Anat Sci J 2013; 10(2):
111-6.
Introduction
Situs inversus is a rare congenital anomaly
characterized by the transposition of the
abdominal organs, viscera and vasculature. When
associated with dextrocardia it is known as situs
inversus totalis. This condition is generally an
autosomal recessive genetic condition. It may or
may not be associated with dextrocardia, also
known as situs inversus totalis [1,2]. Generally,
this rare genetic anomaly is discovered
incidentally, often when a radiographic assessment
of a patient is undertaken, particularly to
investigate blunt abdominal trauma or infection.
Anatomical Sciences Journal 2013, Vol 10, No 2, Pages 111-116
Downloaded from anatomyjournal.ir at 8:27 +0430 on Saturday May 13th 2017
112
R. Ahadi & H. Shamshirband
The condition affects all major structures within
the thorax and abdomen. The organs are simply
transposed through the sagittal plane. The heart is
located on the right side of the thorax; the
stomach and spleen are located on the right side
of the abdomen and the liver and gall bladder on
the left side. The left lung has three lobes and the
right lung two lobes; blood vessels, nerves,
lymphatics and the intestines are also transposed
(Figure 1). If the heart is located on the right side
of the thorax, it is known as situs inversus with
dextrocardia or situs inversus totalis. When the
heart remains in its normal position on the left
side of the thorax, there is a much rarer condition
(1 in 22000 of the general population) known as
situs inversus with levocardia or situs inversus
incompletus. Situs inversus with levocardia or
dextrocardia without situs inversus present with
much higher rates of congenital defects than situs
inversus with dextrocardia.
This report discusses two cases seen among
10000 patients who have referred to the
Shohadaye
Hafte-Tir
Hospital
Imaging
Department for abdominal CT scans over a four
year period.
studies performed in relation to multiple traumas.
This case was particularly interesting because of
the scarcity of this association and the diagnostic
difficulties
and
deferent
approach
in
Jejunostomy.
Genetics and situs in versus
Situs in versus is an autosomal recessive genetic
condition, although it can be X-linked or found in
identical "mirror" twins. In the absence of
congenital heart defects, individuals with situs
inversus are phenotypically unimpaired and can
lead normal healthy lives without any
complications related to their medical condition
(Figure 2).
Situs inversus occurs more commonly with
dextrocardia [4]. A 3%-5% incidence of
congenital heart disease is observed in situs
inversus with dextrocardia, usually with
transposition of the great vessels. Of these
patients, 80% have a right-sided aortic arch. Situs
inversus with levocardia is rare [5] and it is almost
always associated with congenital heart disease
[6-11].
Figure 1. Schematic drawings illustrate the standard
anatomy of situs solitus (A) and the mirror image of situs
inversus (B). The right lung (RL), left lung (LL), right
atrium (RA), and left atrium (LA) are shown [3].
We report a case of situs in versus discovered in
the Medical Imaging Center following imaging
Figure 2. Situs in versus has an autosomal recessive
pattern of inheritance [12].
Anat Sci J 2013, Vol 10, No 2
113
Downloaded from anatomyjournal.ir at 8:27 +0430 on Saturday May 13th 2017
Situs Inversus Totalis
Many people with situs inversus totalis are
unaware of their unusual anatomy until they seek
medical attention for an unrelated condition. The
reversal of the organs may then lead to some
confusion, as many signs and symptoms will be
on the 'wrong' side. For example, if an individual
with situs inversus develops appendicitis, they
will present to the physician with lower left
abdominal pain, since that is where their appendix
lies. Thus, in the event of a medical problem, the
knowledge that the individual has situs inversus
can expedite diagnosis. People with situs inversus
may inform their physicians before an
examination, so the physician can redirect their
search for heart sounds and other signs. But in
people who present to an Emergency Department
in a coma or those with a low GCS the condition
is different, thus wearing a medical identification
tag can help to inform health care providers in the
event the person is unable to communicate.
Situs inversus also complicates organ
transplantations as donor organs will almost
certainly come from situs solitus (normal)
donors,The geometric problems arise from
placing an organ into a cavity shaped in the mirror
image. because heart and liver are chiral [12] For
example, a person with situs inversus who
requires a heart transplant needs all the vessels to
the transplant donor heart reattached to their
existing ones. However, the orientation of these
vessels in a person with situs inversus is reversed,
necessitating steps so that the blood vessels join
properly.
Case report
All study procedures were approved by the
Medical Ethics Committee Shahid Behesht
University of Medical Sciences, Tehran Iran.
Additionally we got verbal informed consent from
bothof two cases.
Case A
A 27-year-old Iranian male presented to the
Emergency Department of Shohadaye Hafte-Tir
Hospital with multiple trauma due to a car
accident. After admission and examination for
assessment of traumatic injuries he was referred
to the Medical Imaging Center for brain, thoracic
and abdominal CT scans and chest and lower
extremity imaging studies (Figure 3).
Figure 3: AP chest radiograph in a 27-year-old Iranian
male with situs inversus and dextrocardia. This image
shows that the cardiac apex (+) points to the right. Rightsided aortic arch (A). The stomach bubble (S) is visible in
the right upper quadrant.
( DDR ,MMD Italy)
The brain CT scan was normal, however there
were FX in the right femur and FX and DX at
base of the metatarsal and in sonography The
hemoperitoneum was reported. We discovered a
situs inversus totalis on the left side of his liver
The heart was located on the right side of the
thorax, the stomach and spleen on the right side
of the abdomen and the liver and gall bladder
on the left side. The patient's left lung had three
lobes, whereas the right lung had two lobes.
Bloodvessels (Aortic arc, carotid artery, SVC
and IVC), nerves, the alimentary tract and
intestines were also transposed. The thoracic,
Anat Sci J 2013, Vol 10, No 2
Downloaded from anatomyjournal.ir at 8:27 +0430 on Saturday May 13th 2017
114
R. Ahadi & H. Shamshirband
abdominal organs and viscera were completely
reversed. Free fluid was observed in his
peritoneal cavity. The patient underwent
surgery to repair a lacerated mesentery. He had
a pulse rate of 88 beats per minute, a blood
pressure of 140/90 mm Hg and his blood group
was type B.
underwent a pre-operative Exanimation, after
which a median laparotomy was performed,
followed by a tracheostomy and Jejunostomy with
foley catheter. The patient was returned to the ICU.
Case B
A 22-year-old Afghani male presented to the
Emergency Department of Shohadaye Hafte-Tir
Hospital with multiple trauma due to a car
accident. After admission and examination for
assessment of the presence of traumatic injuries, he
was sent to the Medical Imaging Center for brain,
thoracic and abdominal CT scans as well as chest
and cervical spine imaging studies (Figures 4,5,6
and 7). We discovered a situs inversus totalis with
the left side liver, The heart was located on the
right side of the thorax, the stomach and spleen on
the right side of the abdomen, and the liver and gall
bladder on the left side of the patient's body. The
patient's left lung had three lobes and the right lung
had two lobes. The blood vessels (Aortic arc,
carotid artery, SVC and IVC), nerves, the
alimentary tract and intestines were also
transposed. The thoracic, abdominal organs and
viscera were completely inversed. According to the
CT scans, the patient had a subarachnoid
hematoma (SAH), intraventricular hematoma
(IVH), a few contusions and edema in his brain.
There was free fluid in his peritoneal cavity. The
patient was transferred to the ICU. His pupils were
asymmetric and unreactive to light. The right pupil
size was 4 mm and the left pupil was 2 mm. He
had a pulse rate of 96 beats per min, blood pressure
of 100/60 mm Hg and his blood group was type
O+. His physical examination was remarkable for
tenderness and fracture of his right clavicle, and
tenderness and fracture on his right superior and
inferior pubic ramuses. In the ICU, he was in a
vegetative condition and after seven days he
Figure 4. AP tomogram of the chest, abdomen and
pelvis with left-sided liver (L) and right-sided heart (H) and
stomach (S). (MDCT aquilion 16 Toshiba)
Figure 5. Axial CT scan slice of situs inversus with leftsided liver (L) and right stomach (S). (MDCT aquilion 16
Toshiba) from two cases
Anat Sci J 2013, Vol 10, No 2
115
Downloaded from anatomyjournal.ir at 8:27 +0430 on Saturday May 13th 2017
Situs Inversus Totalis
Figure 6. Axial CT scan slice of situs inversus with rightsided aorta and heart (H).
(MDCT aquilion 16 Toshiba)
Figure 7. Axial CT scan slice of situs inversus with leftsided aorta. (MDCT aquilion 16 Toshiba)
Discussion
Situs inversus is a positional anomaly that rotates
the abdominal internal viscera. It is known as
situs inversus totalis when associated with a
transposition of the thoracic organs. Situs inversus
is a rare congenital anomaly with an incidence in
the population of only 0.001 to 0.01 [1, 2] with a
male-to-female ratio of 3:2 [13]. Its transmission
mode is autosomal recessive, but its precise
genetic mechanism has yet to be identified [1, 13].
Situs inversus results from a rotation in the
opposite direction of the viscera and organ during
the organogenesis of the embryo [2, 13]. Patients
with situs inversus who present to the Emergency
Department may have diagnostic problems in
physical examination because of their unusual
anatomy, low GCS and unusual localizations of
their pains and symptoms. In patients with situs
inversus who present with multiple traumas,
medical imaging can assist clinicians and
surgeons to arrive at a correct and reliable
diagnosis. Abdominal and chest X-rays,
sonography and CT scan also facilitate a reliable
and early diagnosis if the patient is unaware of his
unusual anatomy and has a low GCS [1, 13, 14].
Medical imaging can also guide the appropriate
therapeutic choice, biopsy, surgical indications
and approaches, and type and location of the
incision in Jujenostomy or other surgical
procedures such as an appendectomy, chest tube
insertion and organ transplantation. Laparoscopy
is useful in these situations as it favors a
minimally invasive surgical approach in
diagnostics and treatment [15]. In patients with
situs inversus totalis who present with an acute
abdomen that suspended of having appendicitis
that involves left iliac localization, the diagnosis is
more complex and difficult. Until 2008, fewer
than 10 cases of appendicitis associated with situs
inversus were reported in the literature [13]. Half
of these reported pain in their right iliac fossa
despite the presence of situs inversus [1].
Informing physicians and surgeons of the
diagnosis of situs inversus can decrease medicals
errors and prevent some of complications that
may arise in assessment and care.
The occurrence of situs inversus is very rare.
Very few cases have been reported in the
literature. This condition poses a diagnostic
problem that can be solved by medical imaging,
particularly by the performance of a body CT
scan and other imaging modalities such as
sonography and laparoscopy. These procedures
allow for early management of the disease and
guidance for the best approaches.
Acknowledgement
This work was performed, in part, by the Medical
Imaging Center of Shohadaye Hafte-Tir Hospital
in Tehran. We express our appreciation to the
staff and manager of this center.
Anat Sci J 2013, Vol 10, No 2
116
R. Ahadi & H. Shamshirband
Downloaded from anatomyjournal.ir at 8:27 +0430 on Saturday May 13th 2017
References
1. Nelson MJ, Pesola GR. Left lower quadrant pain of
unusual cause. J Emerg Med 2001; 20: 241-5.
2. Kassi A, Kouassi J, Souaga K, Koffi E, Kassanyou S.
Appendicite aiguë sur situs inversus: une forme
topographique à ne pas méconnaitre à propos d'un cas.
Médecine d'Afrique noire. 2004;51:429-31.
3. Wilhelm A. Situs Inversus Imaging 2011 [cited 2011
9/23]; Available from: http://emedicine.medscape.
com/article/413679-overview.
4. Maldjian PD, Saric M. Approach to dextrocardia in
adults: review. AJR Am J Roentgenol 2007; 188:
S39-49.
5. Gindes L, Hegesh J, Barkai G, Jacobson JM, Achiron
R. Isolated levocardia: prenatal diagnosis, clinical
importance, and literature review. J Ultrasound Med
2007; 26: 361-5.
6. Douglas YL, Jongbloed MR, den Hartog WC, Bartelings
MM, Bogers AJ, Ebels T, et al. Pulmonary vein and atrial
wall pathology in human total anomalous pulmonary
venous connection. Int J Cardiol 2009; 134: 302-12.
7. Fung TY, Chan DL, Leung TN, Leung TY, Lau TK.
Dextrocardia in pregnancy: 20 years' experience. J
Reprod Med 2006; 51: 573-7.
8. Palumbo E. Neonatal diagnosis of primary ciliary
dyskinesia. Recent advances. Recenti Prog Med 2008;
99: 207-9.
9. Schrott-Fischer A, Rieger G, Morass B, Bitsche M,
Horak E, Riechelmann H, et al. Diagnostics of
primary ciliary dyskinesia. Laryngo-rhino-otologie
2008; 87: 809-20.
10. Van Mierop LH, Eisen S, Schiebler GL.The
radiographic appearance of the tracheobronchial tree
as an indicator of visceral situs. Am J Cardiol 1970;
26: 432-5.
11. Xu BP, Shen KL, Hu YH, Feng XL, Li HM, Lang
ZQ. Clinical characteristics of primary ciliary
dyskinesia in children. Zhonghua Er Ke Za Zhi 2008;
46: 618-22.
12. Gedda L, Sciacca A, Brenci G, Villatico S, Bonanni
G, Gueli N, et al. Situs viscerum specularis in
monozygotic twins. Acta Genet Med Gemellol
(Roma) 1984; 33: 81-5.
13. Huang SM, Yao CC, Tsai TP, Hsu GW. Acute
appendicitis in situs inversus totalis. J Am Coll Surg
2008; 207: 954.
14. Nisolle JF, Bodart E, de Canière L, Bahati M, Michel
L, Trigaux JP. Appendicite aigu d'expression clinique
gauche: apport diagnostique de la tomodensitométrie.
Arch Pediatr 1996; 3: 47-50.
15. Golash V. Laparoscopic management of acute
appendicitis in situs inversus. J Minim Access Surg
2006; 2: 220-1.
Anat Sci J 2013, Vol 10, No 2
Copyright©2013, Iranian society of anatomical sciences. All rights reserved.
Downloaded from anatomyjournal.ir at 8:27 +0430 on Saturday May 13th 2017
Original Article
Anatomical Sciences Journal 2013, Vol 10, No 1, Pages 7-14